Fact-sheet: Segmental arterial mediolysis
Updated on 02/26/2021 at 3:00 PM
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Definition
Rare vascular disease, non-atherosclerotic and non-inflammatory, affecting medium-caliber arteries (predominantly the splanchnic circulation, celiac trunk, and splenic artery ++).
Histology: vacuolization and lysis of the media (= mediolysis), which may lead to arterial dissection, stenosis, or aneurysm.
Clinical features
Variable
Abdominal pain = main symptom
Hemorrhagic shock
CT
CT angiography with arterial vascular phase:
- Regular segmental thickening (segments of normal artery between lesions), uni- or multifocal, of the wall of one or more visceral arteries.
- Segmental aneurysmal dissections along a single artery with a "string of beads" appearance.
May affect all or part of the arterial circumference.
Secondary consequences: intra-abdominal hematoma, visceral ischemia.
Management
Often managed conservatively.
Spontaneously reversible (repair phase with formation of granulation tissue progressively replaced by fibrosis).
Differential diagnosis
Vasculitides:
- inflammatory (Takayasu, Behçet, PAN...)
- non-inflammatory (fibromuscular dysplasia, Ehlers-Danlos syndrome, Marfan syndrome, atherosclerosis)
- infectious (mycotic aneurysm).